Thrombin generation and whole blood viscoelastic assays in the management of hemophilia: current state of art and future perspectives

作者: Guy Young , Benny Sørensen , Yesim Dargaud , Claude Negrier , Kathleen Brummel-Ziedins

DOI: 10.1182/BLOOD-2012-08-378935

关键词: HemostasisImmunologyThrombinInternal medicineCoagulationHematologyBioinformaticsClotting factorClinical researchBlood viscosityBlood coagulation testMedicine

摘要: Hemophilia is a bleeding disorder that afflicts about 1 in 5000 males. Treatment relies upon replacement of the deficient factor, and response to treatment both clinical research practice based subjective parameters such as pain joint mobility. Existing laboratory assays quantify amount factor plasma, which useful diagnostically prognostically. However, these are limited their ability fully evaluate patient’s clot-forming capability. Newer assays, known global provide far more detailed view thrombin generation clot formation have been studied hemophilia for 10 years. They potential offer objective measure hemophilic phenotype well treatment. In particular, patients who develop inhibitors clotting factors whom bypassing agents required hemostasis, opportunity determine interventions where traditional coagulation cannot. this article we review existing literature discuss several controversial issues surrounding assays. Last, vision future uses briefly described.

参考文章(33)
Textbook of hemophilia Blackwell publishing. ,(2005) , 10.1002/9780470987124
C. J. REA, J. H. FOLEY, J. INGERSLEV, B. SØRENSEN, Factor XIII combined with recombinant factor VIIa: a new means of treating severe hemophilia A Journal of Thrombosis and Haemostasis. ,vol. 9, pp. 510- 516 ,(2011) , 10.1111/J.1538-7836.2010.04171.X
Benny Sørensen, Guy Young, Global Laboratory Assays in Hemophilia Textbook of Hemophilia, 3. pp. 328- 334 ,(2014) , 10.1002/9781118398258.CH45
M. GISSEL, M. F. WHELIHAN, L. A. FERRIS, K. G. MANN, G. E. RIVARD, K. E. BRUMMEL-ZIEDINS, The influence of prophylactic factor VIII in severe haemophilia A. Haemophilia. ,vol. 18, pp. 193- 199 ,(2012) , 10.1111/J.1365-2516.2011.02638.X
M. TROSSAËRT, V. REGNAULT, M. SIGAUD, P. BOISSEAU, E. FRESSINAUD, T. LECOMPTE, Mild hemophilia A with factor VIII assay discrepancy: using thrombin generation assay to assess the bleeding phenotype Journal of Thrombosis and Haemostasis. ,vol. 6, pp. 486- 493 ,(2008) , 10.1111/J.1538-7836.2007.02861.X
G. YOUNG, R. ZHANG, R. MILLER, D. YASSIN, D. J. NUGENT, Comparison of kaolin and tissue factor activated thromboelastography in haemophilia. Haemophilia. ,vol. 16, pp. 518- 524 ,(2009) , 10.1111/J.1365-2516.2009.02165.X
B. Sørensen, P. Johansen, K. Christiansen, M. Woelke, J. Ingerslev, Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation. Journal of Thrombosis and Haemostasis. ,vol. 1, pp. 551- 558 ,(2003) , 10.1046/J.1538-7836.2003.00075.X
Dorthe Viuff, Søren Andersen, Brit B. Sørensen, Stefan Lethagen, Optimizing thrombelastography (TEG) assay conditions to monitor rFVIIa (NovoSeven) therapy in haemophilia A patients. Thrombosis Research. ,vol. 126, pp. 144- 149 ,(2010) , 10.1016/J.THROMRES.2010.05.008